Lymphoma, Cancer
The Gluten File


Small bowel adenocarcinoma complicating coeliac disease: a report of three cases and the literature review.
PMID: 23243535  2012

Where gluten-sensitive enteropathy was diagnosed using modern techniques, NHL risk was increased fourfold. At this level, one in 2,000 persons with gluten-sensitive enteropathy develops NHL each year. In addition to EATCL, DLBCL and possibly other subtypes may be linked to these conditions, and these weaker associations could be investigated in large population-based cohorts with biological samples.
Non-Hodgkin lymphoma and gluten-sensitive enteropathy: estimate of risk using meta-analyses.
PMID:21755296  Oct 2011

Conclusion: Early diagnosis and appropriate treatment of the disease, and follow up of family members are crucial to prevent intestinal lymphoma development.
Celiac disease and fulminant T lymphoma detected too late in a 35-year-old female patient: case report
PMID:21875423  Aug  2011

In conclusion, all patients with dermatitis herpetiformis have a gluten-sensitive enteropathy and should be treated with a gluten-free diet. Next to this it is important to notice that patients with celiac disease have an increased risk of developing a small bowel malignancy. Unexplained upper abdominal pain, weight loss and anemia should lead to additional investigations to exclude a small bowel malignancy in these patients. At last, the diagnosis of a small bowel carcinoma is difficult. Together with the radiologist, the optimal techniques for visualization of this malignancy should be considered.
Untreated celiac disease in a patient with dermatitis herpetiformis leading to a small bowel carcinoma.
PMID:22379467  Jan 2012

Evidence has been growing that the pathogenesis of lymphoproliferative disease involves immune processes deregulation. It is believed that antigens or immunological elements can trigger transformation of normal lymphocyte polyclonal population into monoclonal neoplastic disorder--lymphoproliferative disease. Extensive studies point to the link between malignant lymphoma development and autoimmune or inflammatory diseases--namely rheumatoid arthritis, Sjörgen's syndrome, coeliac disease, systemic lupus erythematosus or thyroiditis.
[Lymphoproliferative disease in patients with autoimmune and inflammatory diseases: significance of antigenic stimulation and inflammatory processes].
PMID: 21560455  May 2011

Small Bowel Adenocarcinoma in a Patient with Coeliac Disease: Case Report and Review of the Literature.
PMID: 21060711   Oct 2010

RESULTS: A history of autoimmune disease with localized alimentary tract effects generally increased cancer risks in the organ(s) affected by the autoimmune disease, such as primary biliary cirrhosis and liver cancer (RR, 6.01; 95% confidence interval [CI], 4.76-7.57); pernicious anemia and stomach cancer (RR, 3.17; 95% CI, 2.47-4.07); and ulcerative colitis and small intestine, colon, and rectal cancers (RR, 2.53; 95% CI, 1.05-6.11; RR, 2.06; 95% CI, 1.70-2.48; and RR, 2.07; 95% CI, 1.62-2.64, respectively). In addition, a history of celiac disease, reactive arthritis (Reiter disease), and systemic sclerosis all were associated significantly with increased risk of esophageal cancer (RR, 1.86-2.86). Autoimmune diseases without localized alimentary tract effects generally were not associated with alimentary tract cancer risk, with the exception of decreased risk for multiple alimentary tract cancers associated with a history of multiple sclerosis.
Autoimmune disease and subsequent risk of developing alimentary tract cancers among 4.5 million US male veterans.
PMID: 21046648
  Nov 2010

In conclusion, our data reveal that the high-level CIM/MSI pathway is typical of CD-associated small bowel carcinomas and indicate that aberrant CpG island methylation links CD and carcinogenesis. The data further suggest that CD should be considered in patients with small bowel adenocarcinoma, particularly when the tumors display MSI.
Small bowel adenocarcinomas in celiac disease follow the CIM-MSI pathway.

PMID: 21042749  Dec  2010

CONCLUSIONS: CD is associated with B- and T-cell lymphomas. Patients with B-cell lymphomas had a better prognosis those with T-cell lymphoma. Therapy is unsatisfactory for enteropathy-type T-cell lymphoma. 
Patients with Celiac Disease and B-Cell Lymphoma Have a Better Prognosis than Those with T-Cell Lymphoma.
PMID: 20851210  Sept 2010

In three EATCL cases, there was history of celiac disease or lactose intolerance and enteropathic changes were noted in the adjacent mucosa. These tumors had CD3+/CD8+/CD56 (+/-)/CD4-/ Granzyme B+ immunophenotype.
Primary intestinal T cell lymphomas in Indian patients - In search of enteropathic T cell lymphoma.

PMID: 20699502
July 2010

Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T lymphocytes, usually presenting as a neoplasm composed of large lymphoid cells and often associated with necrosis and an inflammatory background, including large numbers of histiocytes and eosinophils. Intestinal intraepithelial alpha-beta T-cells have been postulated as the normal-cell counterpart for EATL. EATL is the most common neoplastic complication of coeliac disease. The disease is uncommon in most parts of the world, but is seen with greater frequency in those areas with a high prevalence of coeliac disease, in particular Northern Europe.
Enteropathy-associated T-cell lymphoma.

PMID: 20655757  July 2010

INTRODUCTION: Enteropathy-associated T-cell lymphoma (EATL) is a rare complication of celiac disease (CD) (<1% of lymphoma). Its prognosis is extremely poor. METHODS: Review of literature on pathogenesis, clinical and therapeutic data through PubMed((R)) processing (up to January 2009). RESULTS: The mean age at diagnosis is 59 years. Complications frequently reveal EATL. Refractory CD (RCD), which is considered as a low-grade intra-epithelial lymphoma, is now well recognized as the possible intermediate between CD and high grade EATL. Median survival is 7 months, whatever disease stage, and 5-year survival remains below 20%. The poor prognosis of EATL is related to frequent disseminated stage of the disease (50%) at diagnosis, multi-stage intestinal involvement (50%), poor performance status and malnutrition, high frequency of complications (cachexia, infections, intestinal perforation, bleeding, obstruction), which delay chemotherapy and chemo-resistance.
[Enteropathy associated-T cell lymphoma: A review on clinical presentation, diagnosis, therapeutic strategies and perspectives.]

PMID: 20598464
  June 2010

Small bowel adenocarcinoma is a rare tumor. These tumors are more often sporadic but there is some predisposing disease (Crohn disease, genetic syndrome and rarely celiac disease). Diagnosis is usually performed at an advanced stage because of non-specific nature of clinical manifestations.
[Small bowel adenocarcinoma.]
PMID: 20537487  May 2010

A common repertoire of autoantibodies is shared by cancer and autoimmune disease patients: Inflammation in their induction and impact on tumor growth.
PMID: 19091462 Dec 2008

INTERPRETATION: Coeliac disease causes a certain increased risk of cancer and early death. It is not an innocent condition. This strengthens the arguments for coeliac patients to adhere to a gluten free diet.
[Cancer risk in coeliac disease]
PMID: 19096486 Oct 2008

RESULTS: At diagnosis, malnutrition, ulcerative jejunitis, and lymphocytic gastritis were more common in patients with RCD II than RCD I (P < .05). Overt lymphomas occurred in 2 patients with RCD I and 16 with RCD II. In the univariate analysis, abnormal IEL phenotype and increased age at diagnosis of RCD were predictive factors for overt lymphoma. Abnormal IEL phenotype (P < .01), clonality (P = .01), and overt lymphoma (P = .001) predicted short survival time. Only abnormal IEL phenotype (P = .03) and overt lymphoma (P = .04) were predictive in the multivariate analysis. The 5-year survival rate was 93% in patients with RCD I and 44% with RCD II. CONCLUSIONS: Patients with RCD II have a much more severe presentation and prognosis than patients with RCD I; <44% of patients with RCD II survive 5 years after diagnosis. Abnormal IEL phenotype is a predictive factor but not a necessary condition for the development of overt lymphoma.
Presentation and Long-term Follow-up of Refractory Celiac Disease: Comparison of Type I with Type II.
PMID: 19014942   Oct 2008

Celiac disease is the most common severe food intolerance in the Western world and is due to gluten ingestion in genetically susceptible children and adults. The key treatment in these patients is a gluten-free diet, because most complications are more common when dietary compliance is poor. The most serious complication of celiac disease is the development of neoplasms (the most common of which is enteropathy-associated T-cell lymphoma). However, a number of reports have indicated an increased prevalence of ulcerative jejunitis and extraintestinal manifestations, including chronic hepatitis, fibrosing lung disease, and epilepsy syndromes. We report the case of a 53-year-old-man with long-standing diarrhea; because celiac disease was not suspected, the patient developed celiac-associated T-cell lymphoma and mesenteric panniculitis.
[Intestinal lymphoma and mesenteric panniculitis: complications of undiagnosed celiac disease.]
PMID: 18405487  April 2008

Patients with celiac disease have an increased rate of malignancies that are not limited to lymphomas. Thyroid carcinoma has not previously been associated with celiac disease. However, among a cohort of patients with celiac disease, we identified an increased risk of papillary carcinoma of the thyroid, standard morbidity ratio of 22.52 (95% confidence interval 14.90-34.04; P < .001), compared to United States national surveillance data. These patients were on a gluten-free diet. Only 1 had Hashimoto's thyroiditis, suggesting that mechanisms apart from autoimmune thyroiditis contribute to the increased risk of carcinoma of the thyroid in celiac disease.
Increased Risk of Papillary Thyroid Cancer in Celiac Disease.
PMID: 16957996 Sept 2006